The purpose of this study is to confirm the treatment effects of vatiquinone on the key measures of FA disease progression.

Key Inclusion Criteria:

• mFARS ≥20 to ≤70 at Screening (4 weeks prior to Day 1) and Baseline (Day 1).
• Must be ambulatory as defined by a E7 score of 4 or less on the USS at Screening and Baseline.
• Documentation that participants reached maximum score on items E4, E5, and E3b on the USS of mFARS at Screening and Baseline.
• FA diagnosis (homozygous for guanine-adenine-adenine [GAA] repeat expansion in intron-1 of the frataxin gene), confirmed and documented with GAA repeat length for both alleles by clinical genetic testing.
• Difference in the mFARS score between Screening and Baseline of no more than 4 points.
• Ability to abstain from strong cytochrome P450 (CYP) 3A4 inducers/inhibitors (for example, ketoconazole, rifampin, St. John's wort, grapefruit juice) for at least 4 weeks prior to Baseline and for the duration of the study.

Key Exclusion Criteria:

• Individuals with clinical diagnosis of FA who have point mutations, deletions, or other non-GAA expansion mutations.

• Allergy to vatiquinone, sesame oil, gelatin (bovine and/or porcine), titanium dioxide, or red iron oxide.

• Pregnant or lactating participants or those sexually active participants who are unwilling to comply with proper birth control methods; females of childbearing potential must have a negative pregnancy test at Screening and during the Baseline Visit.

• Comorbidities that may confound study results (for example, fat malabsorption syndrome, other mitochondrial disorder) in the opinion of the investigator.

• Participation in an interventional clinical study or received investigational drug (other than SKYCLARYS® [omaveloxolone]) within 60 days prior to Screening. Participants may be screened after the exclusionary period of 60 days has passed.

• Current use of omaveloxolone. Previous use of omaveloxolone will be allowed if:

  1. Use was less than 3 cumulative months and participants have been off treatment for >30 days.
  2. Use was less than 6 cumulative months and participants have been off treatment for >90 days

• Previous or concurrent use of other investigational treatment for FA.

• Participation in any cell or gene therapy-based treatment for FA.

• Participation in an ongoing study for vatiquinone or current or previous use of vatiquinone.

• Illicit drug use 30 days prior to Screening and during the study.

Note: Other protocol-defined inclusion and exclusion criteria may apply.